Dementia with Lewy bodies
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2008-06-22T16:04:33Z
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{{refimprove|date=May 2008}}
{{Infobox_Disease
| Name = Dementia with Lewy bodies
| Image =
| Caption =
| DiseasesDB = 3800
| ICD10 = {{ICD10|G|31|8|g|30}}
| ICD9 = {{ICD9|331.82}}
| ICDO =
| OMIM =
| MedlinePlus =
| eMedicineSubj = neuro
| eMedicineTopic = 91
| MeshID = D020961
}}
'''Dementia with Lewy bodies''' is sometimes described as second only to [[Alzheimer's disease]] as a cause of [[dementia]].<ref name="pmid12489913">{{cite journal |author=Heidebrink JL |title=Is dementia with Lewy bodies the second most common cause of dementia? |journal=J Geriatr Psychiatry Neurol |volume=15 |issue=4 |pages=182–7 |year=2002 |pmid=12489913 |doi= |url=}}</ref><ref name="pmid16267179">{{cite journal |author=Zaccai J, McCracken C, Brayne C |title=A systematic review of prevalence and incidence studies of dementia with Lewy bodies |journal=Age Ageing |volume=34 |issue=6 |pages=561–6 |year=2005 |month=November |pmid=16267179 |doi=10.1093/ageing/afi190 |url=http://ageing.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=16267179}}</ref> Current estimates are that about 60-to-75% of diagnosed dementias are of the Alzheimer's and mixed (Alzheimer's and vascular dementia) type, 10-to-15% are Lewy Bodies type, with the remaining types being of an entire spectrum of dementias including [[frontotemporal lobar degeneration]], [[Korsakoff's syndrome|alcoholic dementia]], pure [[vascular dementia]], etc.
==Causes==
The causes are not yet well understood, but a locus at [[PARK11]] has been described.<ref name="pmid17681982">{{cite journal |author=Bogaerts V, Engelborghs S, Kumar-Singh S, ''et al'' |title=A novel locus for dementia with Lewy bodies: a clinically and genetically heterogeneous disorder |journal=Brain |volume=130 |issue=Pt 9 |pages=2277–91 |year=2007 |month=September |pmid=17681982 |doi=10.1093/brain/awm167 |url=http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=17681982}}</ref>
==Presentation==
Dementia with Lewy Bodies (DLB) exhibits clinical overlap between Alzheimer's disease and [[Parkinson's disease]]. Pathologically, it is characterized by development of abnormal proteinaceous ([[alpha-synuclein]]) cytoplasmic inclusions, called [[Lewy body|Lewy bodies]], throughout the brain. These inclusions have similar structural features to "classical" Lewy Bodies seen subcortically in Parkinson's disease. Additionally, there is a loss of [[dopamine]]-producing neurons (in the [[substantia nigra]]) similar to that seen in Parkinson's disease, and a loss of [[acetylcholine]]-producing neurons (in the [[Basal nucleus of meynert|basal nucleus of Meynert]] and elsewhere) similar to that seen in Alzheimer's disease. Cerebral atrophy (or shrinkage) also occurs as the [[cerebral cortex]] degenerates. Autopsy series have revealed that the pathology of DLB is often concomitant with the pathology of Alzheimer's disease. That is, when Lewy Body inclusions are found in the cortex, they often co-occur with Alzheimer's disease pathology found primarily in the [[hippocampus]], including: [[neurofibrillary tangles]] (abnormally phosphorylated [[tau protein]]), senile plaques (deposited beta-amyloid protein), and [[granulovacuolar degeneration]].
Within DLB, the loss of cholinergic (acetylcholine-producing) neurons is thought to account for the degradation of cognitive and emotional functioning as in Alzheimer's disease, while the loss of dopaminergic (dopamine-producing) neurons is thought to account for the degradation of motor control as in Parkinson's disease. Thus, DLB is similar in some ways to both the dementia resulting from Alzheimer's disease and Parkinson's disease. In fact, it is often confused in its early stages with Alzheimer's disease and/or vascular dementia ([[multi-infarct dementia]]). The overlap of neuropathologies and presenting symptoms (cognitive, emotional, and motor) may make an accurate differential diagnosis difficult to reach.
==History==
Dementia with Lewy Bodies is not a [[DSM-IV]] recognized diagnosis. (Note: DSM-IV was published in 1994.) In 1996, a consortium of scientists initially proposed and later revised diagnostic guidelines. Central features of DLB include progressive cognitive decline, typically with impairments in memory, visual-spatial abilities, and/or attention.
==Clinical features==
Core features include fluctuating cognition with variations in attention and alertness, recurrent visual [[hallucination]]s (typically early in the disease), and motor features of parkinsonism. DLB patients also often experience repeated falls, syncope (fainting), transient loss of consciousness, and hypersentivity to neuroleptic medications. Generally, DLB is diagnosed when cognitive symptoms develop within a year or two of movement disorder/Parkinsonian symptoms. Recent research suggests that presence of sleep disturbance may also be useful in differentiating DLB from other forms of dementia.
==Treatment==
The treatment of DLB, as with Parkinson's disease, involves striking a balance between treating the motor and emotive/cognitive symptoms. Treatment of the movement portion of the disease can typically result in worsening hallucinations and psychosis, while treatment of the hallucinations and psychosis can result in worsening movement symptoms. The use of [[cholinesterase inhibitor]]s represents the treatment of choice. This improves symptoms, but does not cure the disease. The use of [[memantine]] may be recommended, and may represent a means to slow or prevent the decline of cognitive function, although strong evidence to support or disprove this is lacking.
==Nomenclature==
Dementia with Lewy bodies (DLB) is also known under a variety of other names including, '''Lewy Body dementia''' (LBD), '''Diffuse Lewy Body disease''' (DLBD), '''Cortical Lewy Body disease''' (CLBD), and '''Senile dementia of Lewy type'''. All incorporate the name Lewy, as Dr. [[Frederic Lewy]] (1885-1950) was first to discover the abnormal protein deposits ("Lewy Body inclusions") in the early 1900s.<ref>{{WhoNamedIt|synd|2499|Lewy body dementia}}</ref>
==References==
<references/>
==External links==
*[http://www.med.uni-muenchen.de/haass Laboratory for Alzheimer's and Parkinson's Disease Research - Prof. Dr. Christian Haass]
* {{NINDS|dementiawithlewybodies}}
*[http://www.sciencedaily.com/news/mind_brain/dementia/ Dementia Research News from ScienceDaily]
*[http://www.lewybodydementia.org/ Lewy Body Dementia Association, Inc.]
*[http://www.lewybody.org/ The Lewy Body Society, UK and Europe's only dementia with Lewy bodies charity.]
*[http://www.ftdsupportforum.com/ FTD Support Forum]
*[http://www.ftd-picks.org/ The Association for Frontotemporal Dementias]
*[http://memory.ucsf.edu UCSF Memory and Aging Center] - [http://memory.ucsf.edu/resources.html#ftd FTD Resources] and [http://memory.ucsf.edu/Education/Disease/ftd.html FTD Info]
*Mayo Clinic - [http://www.mayoclinic.com/invoke.cfm?id=AN00541 FTD Info]
*[http://www.pdsg.org.uk/ Pick's Disease Support Group Online]
*[http://www.ftdsupport.com/ Frontotemporal Dementia Caregiver Support Center]
{{Diseases of the nervous system}}
[[Category:Neurological disorders]]
[[Category:Cognitive disorders]]
[[de:Lewy-Körperchen-Demenz]]
[[es:Demencia de cuerpos de Lewy]]
[[nl:Lewy Body-dementie]]
[[pl:Otępienie z ciałami Lewy'ego]]
[[pt:Demência com corpos de Lewy]]
[[fi:Lewyn kappale -tauti]]